Efficacy of Anti-TNFα in Severe and Refractory Neuro-Behcet Disease

Abstract

To report the safety and efficacy of anti-tumor necrosis factor α (TNFα) therapy in severe and refractory neuro-Behçet disease (NBD) patients.Observational, multicenter study including 17 BD patients (70.6% of male, with a median age of 39.3 [24–60] years), with symptomatic parenchymal NBD, refractory to previous immunosuppressant and treated with anti-TNFα (infliximab 5 mg/kg [n = 13] or adalimumab [n = 4]). Complete remission was defined by the disappearance of all neurological symptoms and by the improvement of radiological abnormalities at 12 months.Overall improvement following anti-TNF was evidenced in 16/17 (94.1%) patients including 6 (35.3%) complete response and 10 (58.8%) partial response. The median time to achieve remission was 3 months (1–6). The median Rankin score was 2 (1–4) at the initiation of anti-TNFα versus 1 (0–4) at the time of remission (P = 0.01). Corticosteroids have been stopped in 4 (23.5%) patients, and reduced by more than 50% as compared with the dosage at baseline in 10 (58.8%) patients. Side effects occurred in 23.5% of patients and required treatment discontinuation in 17% of cases.TNF blockade represents an effective therapeutic approach for patients with severe and refractory NBD, a difficult to treat population.

Adalimumab‐Based Treatment Versus Disease‐Modifying Antirheumatic Drugs for Venous Thrombosis in Behçet's Syndrome

“…Vascular involvement in BS represents a clinical issue in terms of morbidity and mortality , and optimal clinical management still remains a matter of debate . Anti‐TNF agents are increasingly reported as the treatment of choice for involvement of different organs in BS ; nevertheless, only few data are available on the role of TNF inhibition in BS patients with vascular involvement .…”

Treating the Different Phenotypes of Behçet's Syndrome

“…Correspondence analysis; male, eye disease, HLA-B51 (+), neurologic involvement Azathioprine Clinical trial: (11,36) Observational evidence (for azathioprine alone or in combination): (37,38) Anti TNF-α Interventional study (for infliximab): (39,40) Observational studies (for infliximab): (41,42) Clinical trials (for adalimumab): (43,44) Observational studies (for adalimumab): (41,45,46) Cyclophosphamide Observational study: (47-49)…”

The associations between interleukin 10 polymorphisms and susceptibility to autoimmune uveitis – a meta-analysis

“…In addition, pro-inflammatory cytokines influence autoimmune uveitis, and blocking agents of the pro-inflammatory cytokine pathway, especially TNF-α inhibitors, have been used to treat this disorder [2,5,24,25]. TNF-α blocking agents are reportedly effective in treating AS, BD, and sarcoidosis accompanying uveitis [26][27][28]. As a result, IL-10 may down-regulate TNF-α production and has been correlated with disease severity [25][26][27][28][29]; thus, IL-10 can affect disease severity and the treatment response of uveitis.…”

“…TNF-α blocking agents are reportedly effective in treating AS, BD, and sarcoidosis accompanying uveitis [26][27][28]. As a result, IL-10 may down-regulate TNF-α production and has been correlated with disease severity [25][26][27][28][29]; thus, IL-10 can affect disease severity and the treatment response of uveitis.…”